1-deoxynojirimycin has been researched along with Lysosomal Enzyme Disorders in 20 studies
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 0 (0.00) | 18.7374 |
| 1990's | 0 (0.00) | 18.2507 |
| 2000's | 12 (60.00) | 29.6817 |
| 2010's | 6 (30.00) | 24.3611 |
| 2020's | 2 (10.00) | 2.80 |
| Authors | Studies |
|---|---|
| Benjamin, ER; Boyd, RE; Khanna, R; Lee, G; Rybczynski, P; Valenzano, KJ; Wustman, BA | 1 |
| Barril, X; Bellotto, M; Bergmann, TJ; Cubero, E; Delgado, A; Fregno, I; García-Collazo, AM; Molinari, M; Morales, S; Pérez-Carmona, N; Pons-Vizcarra, M; Ruano, A; Rudinskiy, M; Soldà, T | 1 |
| Andreotti, G; Bräuer, AU; Cimmaruta, C; Citro, V; Cozma, C; Cubellis, MV; Ernst, M; Fuellen, G; Giese, AK; Haake, LR; Hermann, A; Knospe, AM; Koczan, D; Lukas, J; Rolfs, A; Seemann, S; Struckmann, S | 1 |
| Anderson, K; Griffiths, TD; Lad, M; Thomas, RH | 1 |
| Kaminsky, P; Lidove, O | 1 |
| Balter, R; Banzato, C; Bordugo, A; Dardis, A; Degani, D; Giordano, G; Gugelmo, G; Maines, E; Tadiotto, E | 1 |
| Alves, S; Coutinho, MF; Santos, JI | 1 |
| Baris, H; Cohen, IJ; Mistry, PK; Sands, MS | 1 |
| Butters, TD; Dwek, RA; Jeyakumar, M; Platt, FM | 1 |
| Butters, TD; Dwek, RA; Platt, FM | 1 |
| Cox, TM | 1 |
| Lachmann, RH | 1 |
| Platt, FM; Svanborg, C; Svensson, M | 1 |
| Jakóbkiewicz-Banecka, J; Wegrzyn, A; Wegrzyn, G | 1 |
| Pastores, GM | 1 |
| Jeyakumar, M; Platt, FM | 1 |
| Vellodi, A; Winchester, B; Young, E | 1 |
| Adam, A; Butters, TD; Dwek, RA; Mellor, HR; Platt, FM | 1 |
| Proia, RL; Tifft, CJ | 1 |
| Lachmann, RH; Platt, FM | 1 |
14 review(s) available for 1-deoxynojirimycin and Lysosomal Enzyme Disorders
| Article | Year |
|---|---|
Pharmacological chaperones as therapeutics for lysosomal storage diseases.
Topics: Cell Line; Glycolipids; Humans; Lysosomal Storage Diseases; Molecular Chaperones; Structure-Activity Relationship | 2013 |
[Current therapeutic strategies in lysosomal disorders].
Topics: 1-Deoxynojirimycin; Codon, Nonsense; Codon, Terminator; Cystine Depleting Agents; Enzyme Inhibitors; Enzyme Replacement Therapy; Gene Expression Regulation; Hematopoietic Stem Cell Transplantation; Humans; Imino Pyranoses; Lysosomal Storage Diseases; Mutant Proteins; Renal Insufficiency | 2014 |
Less Is More: Substrate Reduction Therapy for Lysosomal Storage Disorders.
Topics: 1-Deoxynojirimycin; Enzyme Replacement Therapy; Gaucher Disease; Genistein; Humans; Lysosomal Storage Diseases; Mucopolysaccharidoses; Niemann-Pick Disease, Type C | 2016 |
Glycosphingolipid lysosomal storage diseases: therapy and pathogenesis.
Topics: 1-Deoxynojirimycin; Animals; Bone Marrow Transplantation; Chemotherapy, Adjuvant; Disease Models, Animal; G(M2) Ganglioside; Gangliosides; Glucosylceramides; Glucosyltransferases; Glycosphingolipids; Humans; Lysosomal Storage Diseases; Lysosomes; Mice; Models, Biological; Models, Chemical; Morpholines; Sandhoff Disease; Tay-Sachs Disease; Treatment Outcome | 2002 |
New therapeutics for the treatment of glycosphingolipid lysosomal storage diseases.
Topics: 1-Deoxynojirimycin; Animals; Disease Models, Animal; Drug Therapy, Combination; Enzyme Inhibitors; Gaucher Disease; Glycosphingolipids; Humans; In Vitro Techniques; Lysosomal Storage Diseases; Mice | 2003 |
Substrate reduction therapy for lysosomal storage diseases.
Topics: 1-Deoxynojirimycin; Enzyme Inhibitors; Glucosylceramidase; Glucosylceramides; Glycoside Hydrolases; Glycosphingolipids; Humans; Lysosomal Storage Diseases; Substrate Specificity | 2005 |
Miglustat: substrate reduction therapy for glycosphingolipid lysosomal storage disorders.
Topics: 1-Deoxynojirimycin; Animals; Clinical Trials as Topic; Drug Evaluation, Preclinical; Enzyme Inhibitors; Gaucher Disease; Glucosyltransferases; Glycosphingolipids; Humans; Lysosomal Storage Diseases | 2006 |
Glycolipid receptor depletion as an approach to specific antimicrobial therapy.
Topics: 1-Deoxynojirimycin; Animals; Bacterial Adhesion; Escherichia coli Infections; Glycosphingolipids; Humans; Immunity, Innate; Lysosomal Storage Diseases; Receptors, Cell Surface; Urinary Tract Infections | 2006 |
Substrate deprivation therapy: a new hope for patients suffering from neuronopathic forms of inherited lysosomal storage diseases.
Topics: 1-Deoxynojirimycin; Animals; Enzyme Inhibitors; Gaucher Disease; Genistein; Humans; Lysosomal Storage Diseases; Lysosomes; Mucopolysaccharidosis III; Niemann-Pick Diseases; Sandhoff Disease; Tay-Sachs Disease | 2007 |
Miglustat: substrate reduction therapy for lysosomal storage disorders associated with primary central nervous system involvement.
Topics: 1-Deoxynojirimycin; Animals; Brain; Brain Diseases; Enzyme Inhibitors; Humans; Lysosomal Storage Diseases | 2006 |
Substrate reduction therapy.
Topics: 1-Deoxynojirimycin; Animals; Blood-Brain Barrier; Central Nervous System Diseases; Enzyme Inhibitors; Gaucher Disease; Glycoside Hydrolase Inhibitors; Humans; Lysosomal Storage Diseases; Niemann-Pick Disease, Type C; Treatment Outcome | 2008 |
The molecular basis of lysosomal storage diseases and their treatment.
Topics: 1-Deoxynojirimycin; Animals; Endocytosis; Enzyme Inhibitors; Genetic Therapy; Genotype; Humans; Lysosomal Storage Diseases; Lysosomes; Phenotype | 2000 |
Stemming the tide: glycosphingolipid synthesis inhibitors as therapy for storage diseases.
Topics: 1-Deoxynojirimycin; Clinical Trials as Topic; Glycosphingolipids; Humans; Lysosomal Storage Diseases; Propanolamines; Pyrrolidines | 2000 |
Substrate reduction therapy for glycosphingolipid storage disorders.
Topics: 1-Deoxynojirimycin; Animals; Glycosphingolipids; Humans; Lysosomal Storage Diseases; Mice | 2001 |
6 other study(ies) available for 1-deoxynojirimycin and Lysosomal Enzyme Disorders
| Article | Year |
|---|---|
Validation of a highly sensitive HaloTag-based assay to evaluate the potency of a novel class of allosteric β-Galactosidase correctors.
Topics: 1-Deoxynojirimycin; Animals; beta-Galactosidase; Dogs; Gangliosidosis, GM1; Lysosomal Storage Diseases | 2023 |
Proteostasis regulators modulate proteasomal activity and gene expression to attenuate multiple phenotypes in Fabry disease.
Topics: 1-Deoxynojirimycin; alpha-Galactosidase; Biomarkers; Endoplasmic Reticulum; Fabry Disease; Fibroblasts; Gene Expression Regulation, Enzymologic; Humans; Lysosomal Storage Diseases; Lysosomes; Mutation, Missense; Proteasome Endopeptidase Complex; Protein Transport; Proteostasis; Sphingosine | 2020 |
Niemann-Pick type C: contemporary diagnosis and treatment of a classical disorder.
Topics: 1-Deoxynojirimycin; Adult; Female; Humans; Lysosomal Storage Diseases; Neurodegenerative Diseases; Niemann-Pick Disease, Type C; Skin; Treatment Outcome | 2019 |
A neonate with abdominal distension and failure to thrive.
Topics: 1-Deoxynojirimycin; Abdomen; Failure to Thrive; Glycoside Hydrolase Inhibitors; Humans; Hydrops Fetalis; Infant, Newborn; Lysosomal Storage Diseases; Male; Treatment Outcome | 2017 |
Overcoming the Next Barriers to Successful Therapy.
Topics: 1-Deoxynojirimycin; Administration, Oral; Cellulose; Daucus carota; Drug Delivery Systems; Drug Discovery; Enzyme Inhibitors; Enzyme Replacement Therapy; Fabry Disease; Gaucher Disease; Humans; Lysosomal Storage Diseases; Mucolipidoses; Pyrrolidines; Rare Diseases | 2016 |
High-performance cation-exchange chromatography and pulsed amperometric detection for the separation, detection, and quantitation of N-alkylated imino sugars in biological samples.
Topics: 1-Deoxynojirimycin; Animals; Brain; Carbohydrates; Cations; Chromatography, Ion Exchange; Electrochemistry; Liver; Lysosomal Storage Diseases; Mice; Mice, Inbred C57BL; Models, Chemical; Time Factors | 2000 |